860952P

Avanti

Lyso GB3 (synthetic)

powder

Empirical Formula (Hill Notation):
C36H67NO17
分子量:
785.91
价格与库存信息目前不能提供

form

powder

packaging

package of 1 × 500 μg (860952P-500UG)

mfr. no.

Avanti Polar Lipids

application(s)

thin layer chromatography (TLC): 99%

shipped in

dry ice

storage temp.

−20°C

SMILES string

O[C@H]1[C@H](OC[C@H](N)[C@H](O)/C=C/CCCCCCCCCCCCC)O[C@H](CO)[C@@H](O[C@@H]2O[C@H](CO)[C@@H]([C@H](O)[C@H]2O)O[C@H]3O[C@H](CO)[C@@H]([C@H](O)[C@H]3O)O)[C@@H]1O

General description

Globotriaosylsphingosine (Lyso GB3) is a deacylated form of globotriaosylceramide. It is a circulating bioactive glycolipid.

Application

Globotriaosylsphingosine (Lyso GB3) has been used as a standard in generating calibration curve via ultra-performance liquid chromatography (UPLC) method.

Biochem/physiol Actions

Globotriaosylsphingosine (Lyso GB3) is a potential biomarker of Fabry disease.

Packaging

2 mL Amber Serum Vial with Stopper and Crimp Cap (860952P-500UG)

WGK Germany

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Lyso-Gb3 activates Notch1 in human podocytes
Sanchez N, et al.
Human Molecular Genetics, 24(20), 5720-5732 (2015)
The Fabry disease-associated lipid Lyso-Gb3 enhances voltage-gated calcium currents in sensory neurons and causes pain
Choi L, et al.
Neuroscience Letters, 594, 163-168 (2015)
Rui Quinta et al.
Gene, 536(1), 97-104 (2013-12-18)
Fabry disease is an X-linked lysosomal storage disease (LSD) caused by deficient activity of α-Galactosidase A (α-Gal A). As a result, glycosphingolipids, mainly globotriaosylceramide (Gb3), progressively accumulate in body fluids and tissues. Studies aiming at the identification of secondary lipid...

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